Advances in Hematology最新文献

Patients with multiple myeloma (MM) and secondary immunodeficiency (SID) are at risk of infection-related morbidity and mortality owing to the disease process and treatment toxicity. This retrospective cohort study used anonymized data from the Optum-Humedica database (October 2015-March 2020) to assess the burden of infection in patients with MM and SID versus patients without SID. Patients aged ≥ 18 years with confirmed MM were assigned to SID and no-SID cohorts using an algorithm considering serum IgG levels < 5.0 g/L, hypogammaglobulinemia diagnosis codes, and ≥ 1 major infection. Patients with SID were stratified into those treated or not treated with immunoglobulin replacement therapy (IgRT and no-IgRT cohorts). At 12 months follow-up, a greater proportion of the SID cohort than the no-SID cohort experienced infections (58.9% vs. 31.3%; p < 0.001), severe infections (29.8% vs. 10.6%, p < 0.001), and infection-related hospitalizations (26.9% vs. 9.1%; p < 0.001). Bacterial infections were the most common infection type (SID cohort, 49.2%; no-SID cohort, 26.1%; IgRT cohort, 70.4%; no-IgRT cohort, 45.1%). Use of anti-infectives and healthcare resource utilization was higher in the SID cohort than in the no-SID cohort. Median overall survival was shorter in the SID versus no-SID cohort (12.6 vs. 14.7 months; p < 0.001) and was similar in the IgRT versus no-IgRT cohort (8.5 vs. 12.5 months; p = 0.446). Among patients with MM and SID, the higher infection burden in patients treated with IgRT than no-IgRT suggests the IgRT cohort was a more vulnerable population. A better understanding of SID burden may improve outcomes for patients with MM.

Background: Primary warm autoimmune hemolytic anemia (PWAIHA) is a subtype of autoimmune hemolytic anemia (AIHA) characterized by premature destruction of red blood cells (RBCs) due to autoantibodies, typically immunoglobulin G (IgG), the cornerstone of which is a positive direct antiglobulin test. The indirect antiglobulin test (IAT) is used as a screening test to detect common and clinically significant RBC alloantibodies in patient serum.

Objective: This study aimed to analyze the prevalence, clinical characteristics, and impact of positive IAT in adult patients with PWAIHA at the time of diagnosis, as well as the response to corticosteroids as the first-line treatment.

Methods: This single-center retrospective case-control study was conducted between September 2002 and May 2024 at King Abdullah University Hospital. We analyzed data from 80 adult patients diagnosed with PWAIHA, aged a minimum of 16 years. After recording baseline investigations, including IAT and antinuclear antibody (ANA), all patients were treated with corticosteroids as first-line treatment. The response rate and prevalence of ANA were compared between the IAT-positive and -negative PWAIHA groups.

Results: Baseline IAT positivity was found in 65% of our patients. Both groups were comparable in terms of age, sex, and hemoglobin levels. Serum LDH presentations were higher in positive IAT patients, response to corticosteroids was numerically higher in patients with negative IAT (54%) than those with positive IAT (33%), and after tapering steroids, patients with a positive IAT had higher rates of relapses compared with those with negative IAT. Positive ANA was found only in IAT-positive patients (25%), which was statistically significant.

Conclusion: An association between baseline IAT positivity and lower rates of complete response was observed in PWAIHA patients. The presence of IAT serves as a prognostic indicator and aids in the decision-making process regarding treatment options. Furthermore, positive IAT results are linked to a higher prevalence of ANA positivity.

Background: The Hyper-CVAD protocol is a chemotherapy regimen widely used in hematological malignancies. It is considered one of most promising remission-leading treatment option for adults with acute lymphoblastic leukemia (ALL).

Objective: To determine the outcomes of adults with ALL treated with the Hyper-CVAD protocol in Ecuador.

Methods: This multicenter, retrospective, cross-sectional study compared the outcomes of ALL patients aged 15 years and older treated with hyper-CVAD protocol in 8 specialized centers.

Results: 139 patients with ALL treated with the Hyper-CVAD protocol were included. A total of 79 were female (56.8%) and the majority had a B-type phenotype 130 (93.5%). A complete response (CR) was achieved in 64.3%. Relapse was confirmed in 52.7% of those who obtained CR. Negative minimal residual disease (MRD) was achieved in 20% of patients. The median overall survival (OS) was 11 months (95% CI: 7.49-14.50) with a 5-year OS of 14.0%. A total of 12 (8.6%) deceased during induction phase.

Conclusion: Adult patients treated with the Hyper-CVAD protocol in Ecuador achieve rates of CR, MRD, and OS lower than those presented in international cohorts, as well as higher rates of treatment-related toxicity.

Background: CKD is a progressive disorder that is commonly associated with hematological abnormalities.

Objective: The objective of the study was to evaluate the role of hematological biomarkers in predicting CKD progression and related complications in adult patients.

Methods: The retrospective cross-sectional study evaluated hematological parameters such as hemoglobin, RBC, WBC, platelet, and NLR; comorbidities; and CKD stages were recorded for analysis. Statistical analysis methods used included ANOVA, Chi-square, multiple logistic regression, Pearson correlation analyses, and ROC curve analysis. The study was conducted under strict adherence to the principles and guidelines of the Helsinki Declaration (2013).

Results: The mean age was 56.2 ± 14.8 years, with males being 56.7%. About 70.8% of the patients were in CKD Stages 3-5. Anemia was observed in 74.2% of the patients, whose prevalence increased alongside the increase in severity of CKD (p < 0.001). There was a significant decrease in hemoglobin, RBC, and platelet counts with advancing CKD stages, whereas WBC and NLR increased (p < 0.001). Hemoglobin (OR: 0.72; p < 0.001), NLR (OR: 1.43; p = 0.006), and platelet count (OR: 0.98; p = 0.021) were independent predictors of progression to CKD Stage 5. ROC analysis yielded good results for hemoglobin (AUC: 0.81) and NLR (AUC: 0.76) in predicting CKD Stage 5. Hemoglobin and platelet-count levels were significantly correlated with eGFR (r = -0.70 and r = 0.58, respectively).

Conclusion: The performance of hematological biomarkers, mainly hemoglobin and NLR, emerges as reliable predictor of CKD progression and complications. Their assessment as part of the CKD workup may then enhance risk stratification and early intervention.

Introduction: Intravenous immunoglobulin (IVIG) is a weight-based therapy used to treat immune thrombocytopenia (ITP). Although pharmacokinetic data support the use of ideal body weight (IBW)-based dosing, clinical outcomes for this dosing strategy are lacking.

Materials and methods: This retrospective, multicenter chart review was conducted across five institutions compared clinical outcomes and costs for patients with ITP treated with IVIG dosed via actual body weight (ABW) or IBW. Data were collected from November 1, 2019 to April 10, 2024. The primary outcome was early platelet response, and secondary outcomes included length of stay (LOS) and increase in platelet count.

Results: A total of 94 patients were included for analysis. No significant differences were noted between the ABW and IBW groups for early platelet response (59.1% vs. 47.2%, p = 0.466), LOS (9 vs. 6 days, p = 0.111), and the increase in platelet count (54.5 vs. 18.5 × 10⁹/L, p = 0.681). The estimated average cost of IVIG per patient was $5231.87 lower for the IBW group.

Conclusions: The utilization of IBW-based dosing of IVIG for ITP treatment was not associated with a change in early platelet response, LOS, or increase in platelet count compared with ABW-based dosing but was associated with cost savings.

Patients with chronic lymphocytic leukemia (CLL) treated with covalent Bruton's tyrosine kinase inhibitors (BTKi) eventually discontinue treatment, but data on outcomes post-BTKi treatment discontinuation are limited. In this single-institution, retrospective chart review of 104 adult patients with CLL treated with a covalent BTKi between July 2011 and April 2019 and who subsequently discontinued for any reason, the majority of patients (59.9%) received the index BTKi in their first (27.5%) or second (32.4%) line of therapy. Median progression-free survival (PFS) from index BTKi initiation was 3.2 years, and median overall survival (OS) was 8.9 years. There was a notable correlation between PFS and OS (r = 0.79). Following the discontinuation of their last BTKi treatment, over half of these patients received subsequent therapies (80.6% of whom received regimens containing B-cell lymphoma 2 inhibitors [BCL2i]). Of the patients who received BCL2i-containing therapy after index BTKi treatment, 77.8% achieved an overall response to BCL2i at first clinical assessment. However, 73.8% of the patients exposed to BTKi and BCL2i eventually discontinued BCL2i treatment and 36.1% died, with a median follow-up of 2.6 years from BCL2i initiation. A subset of patients who were exposed to and were found to be relapsed, refractory, resistant, or intolerant to both BTKi and BCL2i-based regimens (n = 25) had high rates of progressive disease at first assessment (15.0%) and death (44.0%). These findings highlight an unmet clinical need for patients with CLL and underscore the urgency to develop effective new strategies to treat those patients who have already received covalent BTKi and BCL2i.

Background: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an inherited disorder caused by a genetic defect in the red blood cell enzyme G6PD, affecting around 500 million people worldwide. The study investigated the optimum Methaemoglobin Reduction Test (MRT) reaction time for diagnosing G6PD deficiency among patients at Asutifi North District Hospital using Monica Cheesbrough protocol and Asutifi North District Hospital Protocol.

Methodology: The study was an experimental study conducted with 643 participants from April, 2024 to July, 2024. The Monica Cheesbrough MRT and the modified approach (Asutifi North Hospital MRT protocols), were compared at various time points (T90, T120, T150, and T180 min). Intraclass Correlation Coefficient (ICC) and Kappa statistics were used to assess reliability and agreement between the results from the two protocols. McNemar's test was utilized to analyse G6PD status differences between the protocols. The prevalence of G6PD deficiency was also determined. Data analyses were done using IBM Statistical Package for the Social Sciences version 26.0 (2019. Armonk, NY: IBM Corp). p-value less than 0.05 was considered statistically significant for all analyses.

Results: T90 ICC values were very low (0.005-0.007), indicating poor agreement. From T120 onwards, ICC values were high (0.967-0.996, p < 0.001), demonstrating excellent reliability. Significant differences in G6PD status were found at T90 (p < 0.001), with diminishing differences at later time points. Kappa values indicated slight agreement at T90 (K = 0.164, p < 0.001) and perfect agreement from T120 onwards (K = 1.000, p < 0.001). The majority of participants 606 (94.2%) had normal G6PD status, 29 (4.5%) had a fully defective enzyme, and 8 (1.2%) had a partially defective enzyme activity.

Conclusion: The study found the optimum MRT reaction to be 120 min. The study also emphasized lesser volumes of blood samples and reagent give accurate results in the optimum time established. These insights can contribute to faster turnaround times and efficient sample and reagent management especially amongst paediatric patients where it is difficult taking very large volumes of blood for testing.

This study describes treatment patterns and clinical and economic outcomes of patients with myelodysplastic syndrome (MDS) by Revised International Prognostic Scoring System (IPSS-R) risk scores. A retrospective cohort study was conducted including adults diagnosed with MDS between 2010 and 2020 within two academic institutions. A total of 369 patients were included. Hypomethylating agents (HMAs) were used as first-line therapy in 81% (n = 230) and 51% (n = 44) of higher-risk (HR) and lower-risk (LR) patients, respectively. Second-line therapy was received by 24% of patients. Complete response was achieved by 11% of HR patients (median duration: 24 months). Median PFS and OS were 9.5 and 18.8 months in the HR cohort and 18.8 and 25.6 months in the LR cohort. Mean MDS-related healthcare charges per patient per month were > 2-fold higher among HR patients compared to LR (p < 0.001). An unmet need exists for first and subsequent lines of therapy.

Introduction: The differentiation and diagnosis of plasma cell (PC) neoplasms (PCNs) such as multiple myeloma (MM) rely on the quantification of clonal PCs in the bone marrow (BM). For monitoring, the International Myeloma Working Group (IMWG) defines stringent response criteria based on the percentage of BM PC. However, BM biopsies are invasive and painful, and often with sampling variability. This study investigates whether routine biomarkers can predict BM trephine (BMT) PC% using multivariate regression.

Methods: A cross-sectional study was conducted at Tygerberg Hospital, South Africa. Data were extracted from the National Health Laboratory Service (NHLS) database. The final dataset included 112 newly diagnosed MM patients with complete biomarker data for training of the partial least squares regression (PLS-R) model. Variables analyzed included SFLC ratio, paraprotein, Hb, calcium, creatinine, and albumin. Statistical methods included correlation analysis, regression modeling, and internal validation.

Results: The cohort had a median age of 61 years and a male-to-female ratio of 1:1.5. PLS-R analysis identified significant predictors of BMT PC%, including SFLC ratio, paraprotein, Hb, and serum albumin. The final model equation showed moderate predictive power (Q ² = 0.410, R ² Y = 0.432). Spearman correlation analysis showed a moderate positive relationship (p = 0.585) between predicted and actual BMT PC%. Linear regression analysis also confirmed that BMA PC% (R ² = 0.489) was a stronger predictor of BMT PC% than flow cytometry PC% (R ² = 0.184).

Conclusion: This study provides proof of concept for the use of biochemical markers to predict BMT PC% and offers a less invasive alternative for PC quantification in PCN. Standardization of sampling and measurement of biomarkers is essential for the refinement of these predictive models. Future multicenter studies should include prospective data collection to improve model accuracy and clinical applicability.

Background: Although blood donation saves lives, it may be associated with adverse reactions. These reactions may be immediate or delayed and may be a factor in donor nonreturn. However, there is limited knowledge about blood donation-related adverse reactions and risk factors specific to the Ghanaian context.

Aim: To determine the prevalence of adverse blood donation reactions and associated risk factors among successful blood donors at a tertiary hospital setting.

Materials and methods: This mixed-methods research (May-June 2024) used observational techniques and semistructured questionnaires to investigate adverse reactions during and after blood donation. The study recruited 279 participants (241 mobile session donors and 38 in-house donations) in a tertiary setting blood collection center. Bivariate logistic regression analysis was performed to evaluate the influence of some donor demographics on adverse reactions.

Results: Overall, the prevalence of immediate adverse reaction was 44.4%; 51.4%, 36.5%, and 12.1% were local, vasovagal, and allergies, respectively. Blood donors who experienced adverse events had a statistically significantly lower median age (p = 0.007), lower systolic blood pressure (p = 0.004), lower diastolic pressure (p = 0.007), and lower weight (p = 0.030). Also, 22.1% of adverse donor reactions persisted 24 h postdonation; 38.7%, 58.1%, and 3.2% were local, vasovagal, and allergies, respectively. Bivariate logistics regression analysis showed that 16-19 years (aOR, 2.572, p = 0.477), males (aOR, 1.492, p = 0.125), students (aOR, 2.421, p = 0.325), the mobile session (aOR, 1.063, p = 0.928), and first-time donors (aOR, 1.139, p = 0.690) were associated with nonstatistically significantly increased risk of immediate adverse donor reactions.

Conclusion: There is an urgent need to build staff competencies and operationalize standard operating protocols to enable staff to identify and handle adverse blood donation events quickly. Further studies are needed to understand the factors responsible for the high prevalence of adverse donor reactions to inform improvements in blood donor care.